T/F? GVHD involving liver p/w hepatitic LFT derangment False
usually p/w cholestatic LFTs. DDx: veno-occlusive disease
Hepcidin is involved in control of iron absorption. Which of the following conditions would be a/w high hepcidin. A) Anaemia. B) Iron deficiency. C) Thalassaemia. D) Sideroblastic anaemia. E) Haemochromatosis. F) Chronic inflammation. Both Anaemia
The JAK2 mutation is in what % of PRV
and what % of ET? PRV - 95%
Are the following cytogenetics favourable are unfavourable? inv(16)
Are the following cytogenetics favourable are unfavourable? inv(16); t(8;21); t(15;17)
In ALL, which of the following are favourable? Which are unfavourable? BCR-ABL, MLL rearrangement
Both are unfavourable; they'll do poorly. Need aggressive post-remission therapies
What's imatinib, and how is it used?
It's molecular targeted therapy at tyrosine kinase, added on to chemoTx, and has drastically improved prognosis of CML
The cytogenetic marker, 'del 5q' is found in what condition?
Subtype of MDS. This type is responsive to lenalidomide.
In which part of the GIT is iron absorbed?
Where in the body is transferrin synthesised?
There are two types of transferrin receptors (types 1 & 2). Where are they located, and which is implicated in HH?
Type 1 - major binding port for iron, found on most cells. Binds transferrin 30x stronger than TfR2.
What is ferritin?
It's a storage facility for iron. Ferritin consists of an apoprotein shell with light & heavy chain subunits, which surround a core of iron atoms.
How do ferritin levels help us work out how much iron is in the body?
Cells excrete the same amount of ferritin storage units into the blood stream as they currently have inside them, and so checking the ferritin levels in the blood gives us an indication of how many ferritin 'storage units' there are within the cells, and from there, we can guess what amount of iron is present in the cell
What's the main driver of ferritin production?
Iron in cells.
What's the link between hepcidin and ferroportin?
Hepcidin causes internalisation of ferroportin, and so iron has no way of leaving the cell (it's only exit is via ferroportin). When body has low iron in storage, hepcidin expression is reduced (to allow ferroportin to stay on cell membrane & get iron out of cell into blood); and vice versa
Which type of HD carries the best prognosis?
T/F? With regards to Hodgkins Disease, the presence of B symptoms implies a better prognosis
False - worse prognosis
Name 2 poor prognostic factors for HD?
As per NEJM 1998
Name an alkylating agent
Cyclophosphamide - causes cross-linking of DNA. S/Es: haemorrhagic cystitis, myelosuppression, transitional cell carcinoma
Name a S/E of bleomycin
Name a S/E of doxorubicin
Name 2 anti-metabolite drugs
Name a chemoTx agent that causes peripheral neuropathy
Name 2 modalities which may help improve metastatic bone pain
Name cytogenetic abnormalities for: a) CML, b) APML, c) Burkitt's, d) Mantle cell lymphoma
a) CML - t(9:22) - Philadelphia chromsome; BCR-AbL
What's the first-line treatment for pyoderma gangrenosum?
Oral steroids; if not resolving, try immunosuppressive therapy (ciclosporin, infliximab)
Name 2 causes of warm AIHA
AID, eg SLE
Name 2 causes of cold AIHA
Neoplasia, eg lymphoma
How do you treat warm AIHA?
How do you diagnosis hereditary haemorrhagic telangiectasia?
Aka Osler-Weber-Rendu syndrome, is an AD condition characterised by multiple telangiectasia over skin / MMs. If you meet 2/4 of following = 'possible' diagnosis, if you meet 3/4 or 4/4 = 'definite' diagnosis. 1) Recurrent, spont. epistaxis. 2) Telangiectases (lips, mouth, fingers, nose). 3) AVMs in GIT, hepatic, cerebral, spinal, lungs. 4) FHx (1st degree relative) with HHT